Size frequency distribution of prion protein (PrP) aggregates in variant Creutzfeldt-Jakob disease (vCJD)

J Neural Transm (Vienna). 2005 Nov;112(11):1565-73. doi: 10.1007/s00702-005-0296-2. Epub 2005 Mar 23.

Abstract

The frequency distribution of aggregate size of the diffuse and florid-type prion protein (PrP) plaques was studied in various brain regions in cases of variant Creutzfeldt-Jakob disease (vCJD). The size distributions were unimodal and positively skewed and resembled those of beta-amyloid (A beta) deposits in Alzheimer's disease (AD) and Down's syndrome (DS). The frequency distributions of the PrP aggregates were log-normal in shape, but there were deviations from the expected number of plaques in specific size classes. More diffuse plaques were observed in the modal size class and fewer in the larger size classes than expected and more florid plaques were present in the larger size classes compared with the log-normal model. It was concluded that the growth of the PrP aggregates in vCJD does not strictly follow a log-normal model, diffuse plaques growing to within a more restricted size range and florid plaques to larger sizes than predicted.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Alzheimer Disease / diagnosis
  • Alzheimer Disease / pathology
  • Brain / pathology*
  • Brain / physiopathology
  • Creutzfeldt-Jakob Syndrome / diagnosis*
  • Creutzfeldt-Jakob Syndrome / physiopathology
  • Diagnosis, Differential
  • Disease Progression
  • Female
  • Humans
  • Male
  • Middle Aged
  • Models, Neurological
  • Neuropil / pathology*
  • Plaque, Amyloid / metabolism
  • Plaque, Amyloid / pathology
  • Prions / metabolism*
  • Statistical Distributions

Substances

  • Prions